A Case of Shwachman's Syndrome (Pancreatic insufficiency and bone marrow dysfunction)

—Evaluation of Hemopoietic Disorder by in vitro Culture Technique—

Abstract

A ten-month-old boy with Shwachman's syndrome (chronic neutropenia and exocrine pancreatic insufficiency) was evaluated.
Hemopoietic disorder in this case was studied by in vitro culture technique. An assay of bone marrow granulocyte-macrophage precursor (CFU-C) in a double agar culture system demonstrated its reduced number. Production of colony stimulating factor (CSF) from patient's peripheral blood leukocytes appeared normal when tested on normal marrow. No serum inhibitor against CFU-C could be demonstrated.
The reduced number of erythroid progenitor cells (CFU-E) was shown in a plasma clot culture system.
These findings suggested that the bone marrow dysfunction in this case might be resulted from a stem cell disorder.